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3. The importance of early immunotherapy in patients with faciobrachial dystonic seizures

Author

Thompson, Julia, Bi, Mian, Murchison, Andrew G, Makuch, Mateusz, Bien, Christian G, Chu, Kon, Farooque, Pue, Gelfand, Jeffrey M, Geschwind, Michael D, Hirsch, Lawrence J, Somerville, Ernest, Lang, Bethan, Vincent, Angela, Leite, Maria I, Waters, Patrick, Irani, Sarosh R, Dogan-Onugoren, Müjgan, Rae-Grant, Alexander, Illes, Zsolt, Szots, Monika, Malter, Michael, Widman, Guido, Surges, Rainer, Archibald, Neil, Reid, John, Duncan, Callum, Richardson, Anna, Lilleker, James, Iorio, Rafaelle, Blaabjerg, Morten, Abeler, Karin, and Shin, Y

Subjects
Brain Disorders, Neurosciences, Prevention, Clinical Research, Epilepsy, Neurodegenerative, Rare Diseases, ADAM Proteins, Adult, Aged, Aged, 80 and over, Analysis of Variance, Antibodies, Anticonvulsants, Cognition Disorders, Disabled Persons, Female, Flow Cytometry, Follow-Up Studies, Green Fluorescent Proteins, HEK293 Cells, Humans, Immunotherapy, Intracellular Signaling Peptides and Proteins, Limbic Encephalitis, Male, Middle Aged, Nerve Tissue Proteins, Protein Transport, Proteins, Retrospective Studies, Seizures, Surveys and Questionnaires, Transfection, Young Adult, faciobrachial dystonic seizures, leucine-rich glioma-inactivated 1, seizures, immunotherapy, cognitive impairment, Faciobrachial Dystonic Seizures Study Group, Medical and Health Sciences, Psychology and Cognitive Sciences, Neurology & Neurosurgery
Abstract

Faciobrachial dystonic seizures and limbic encephalitis closely associate with antibodies to leucine-rich glioma-inactivated 1 (LGI1). Here, we describe 103 consecutive patients with faciobrachial dystonic seizures and LGI1 antibodies to understand clinical, therapeutic and serological differences between those with and without cognitive impairment, and to determine whether cessation of faciobrachial dystonic seizures can prevent cognitive impairment. The 22/103 patients without cognitive impairment typically had normal brain MRI, EEGs and serum sodium levels (P < 0.0001). Overall, cessation of faciobrachial dystonic seizures with antiepileptic drugs alone occurred in only 9/89 (10%) patients. By contrast, 51% showed cessation of faciobrachial dystonic seizures 30 days after addition of immunotherapy (P < 0.0001), with earlier cessation in cognitively normal patients (P = 0.038). Indeed, expedited immunotherapy (P = 0.031) and normal cognition (P = 0.0014) also predicted reduced disability at 24 months. Furthermore, of 80 patients with faciobrachial dystonic seizures as their initial feature, 56% developed cognitive impairment after 90 days of active faciobrachial dystonic seizures. Whereas only one patient developed cognitive impairment after cessation of faciobrachial dystonic seizures (P < 0.0001). All patients had IgG4-LGI1 antibodies, but those with cognitive impairment had higher proportions of complement-fixing IgG1 antibodies (P = 0.03). Both subclasses caused LGI1-ADAM22 complex internalization, a potential non-inflammatory epileptogenic mechanism. In summary, faciobrachial dystonic seizures show striking time-sensitive responses to immunotherapy, and their cessation can prevent the development of cognitive impairment.awx323media15681705685001.

Published
2018

4. Protecting Great Barrier Reef resilience through effective management of crown-of-thorns starfish outbreaks

Author

Matthews, Samuel A., primary, Williamson, David H., additional, Beeden, Roger, additional, Emslie, Michael J., additional, Abom, Rickard T. M., additional, Beard, Daniel, additional, Bonin, Mary, additional, Bray, Peran, additional, Campili, Adriana R., additional, Ceccarelli, Daniela M., additional, Fernandes, Leanne, additional, Fletcher, Cameron S., additional, Godoy, Dan, additional, Hemingson, Christopher R., additional, Jonker, Michelle J., additional, Lang, Bethan J., additional, Morris, Sheriden, additional, Mosquera, Enrique, additional, Phillips, Gareth L., additional, Sinclair-Taylor, Tane H., additional, Taylor, Sascha, additional, Tracey, Dieter, additional, Wilmes, Jennifer C., additional, and Quincey, Richard, additional

Published
2024
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5. Immune or Genetic-Mediated Disruption of CASPR2 Causes Pain Hypersensitivity Due to Enhanced Primary Afferent Excitability

Author

Dawes, John M., Weir, Greg A., Middleton, Steven J., Patel, Ryan, Chisholm, Kim I., Pettingill, Philippa, Peck, Liam J., Sheridan, Joseph, Shakir, Akila, Jacobson, Leslie, Gutierrez-Mecinas, Maria, Galino, Jorge, Walcher, Jan, Kühnemund, Johannes, Kuehn, Hannah, Sanna, Maria D., Lang, Bethan, Clark, Alex J., Themistocleous, Andreas C., Iwagaki, Noboru, West, Steven J., Werynska, Karolina, Carroll, Liam, Trendafilova, Teodora, Menassa, David A., Giannoccaro, Maria Pia, Coutinho, Ester, Cervellini, Ilaria, Tewari, Damini, Buckley, Camilla, Leite, M. Isabel, Wildner, Hendrik, Zeilhofer, Hanns Ulrich, Peles, Elior, Todd, Andrew J., McMahon, Stephen B., Dickenson, Anthony H., Lewin, Gary R., Vincent, Angela, and Bennett, David L.

Published
2018
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9. Supplementary Material COTS settlement cue selectivity Doll et al. Biol Lett from Settlement cue selectivity by larvae of the destructive crown-of-thorns starfish

Author

Doll, Peter C., Uthicke, Sven, Caballes, Ciemon F., Diaz-Pulido, Guillermo, Abdul Wahab, Muhammad A., Lang, Bethan J., Jeong, So Young, and Pratchett, Morgan S.

Abstract

Supplementary file containing 1) COTS spawning and larval rearing methodology, 2) coralline algae species information table, 3) Molecuar-based methods for algal species identification, 4) GenBank Accession numbers

Published
2023
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19. NK Cell-mediated Neuroblastoma Cell Lysis is Enhanced by IgG From Patients With Pediatric Opsoclonus-Myoclonus Syndrome

Author

Zar, Tahira, Tschernatsch, Marlene, Hero, Barbara, Lang, Bethan, Preissner, Klaus T., Blaes, Franz, Zar, Tahira, Tschernatsch, Marlene, Hero, Barbara, Lang, Bethan, Preissner, Klaus T., and Blaes, Franz

Abstract

Pediatric opsoclonus-myoclonussyndrome (OMS) is a rare autoimmune disorder of which 50% are associated with neuroblastoma (NB). We investigated whether surface-binding autoantibodies in OMS can enhance natural killer (NK) cell-mediated cytotoxicity in these patients. OMS immunoglobulin G (IgG) bound to NB cell lines and NK cell-mediated cytotoxicity to NB cells was enhanced after preincubation with OMS-IgG, but not IgG from NB without OMS or healthy controls. Activation of NK cells by surface-binding autoantibodies may be an additional mechanism of antitumor immunity in children with NB and OMS.

Published
2021

24. Faciobrachial dystonic seizures: the influence of immunotherapy on seizure control and prevention of cognitive impairment in a broadening phenotype

Author

Irani, Sarosh R., Stagg, Charlotte J., Schott, Jonathan M., Rosenthal, Clive R., Schneider, Susanne A., Pettingill, Philippa, Pettingill, Rosemary, Waters, Patrick, Thomas, Adam, Voets, Natalie L., Cardoso, Manuel J., Cash, David M., Manning, Emily N., Lang, Bethan, Smith, Shelagh J. M., Vincent, Angela, and Johnson, Michael R.

Published
2013
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32. Human autoantibodies specific for the alpha(sub 1A) calcium channel subunit reduce both P-type and Q-type calcium currents in cerebellar neurons

Author

Pinto, Ashwin, Gillard, Samantha, Moss, Fraser, Whyte, Kathryn, Brust, Paul, Williams, Mark, Stauderman, Ken, Harpold, Michael, Lang, Bethan, Newsom-Davis, John, Bleakman, David, Lodge, David, and Boot, John

Subjects
Autoantibodies -- Research, Calcium channels -- Research, Cerebellum -- Research, Neurons -- Physiological aspects, Science and technology
Abstract

The pharmacological properties of voltage-dependent calcium channel (VDCC) subtypes appear mainly to be determined by the [[Alpha].sub.1] pore-forming subunit but, whether P-and Q-type VDCCs are encoded by the same [[Alpha].sub.1] gene presently is unresolved. To investigate this, we used IgG antibodies to presynaptic VDCCs at motor nerve terminals that underlie muscle weakness in the autoimmune Lambert-Eaton myasthenic syndrome (LEMS). We first studied their action on changes in intracellular free [Ca.sup.2+] concentration [[Ca.sup.2+]]i in human embryonic kidney (HEK293) cell lines expressing different combinations of human recombinant VDCC subunits. Incubation for 18 h with LEMS IgG (2 mg/ml) caused a significant dose-dependent reduction in the [K.sup.+]-stimulated [[Ca.sup.2+]]i increase in the [[Alpha].sub.1A] cell line but not in the [[Alpha].sub.1B], [[Alpha].sub.1C], [[Alpha].sub.1D], and [[Alpha].sub.1E] cell lines, establishing the [[Alpha].sub.1A] subunit as the target for these autoantibodies. Exploiting this specificity, we incubated cultured rat cerebellar neurones with LEMS IgG and observed a reduction in P-type current in Purkinje cells and both P- and Q-type currents in granule cells. These data are consistent with the hypothesis that the [[Alpha].sub.1A] gene encodes for the pore-forming subunit of both P-type and Q-type VDCCs.

Published
1998

35. The utility of anti-SOX2 antibodies for cancer prediction in patients with paraneoplastic neurological disorders

Author

Maddison, Paul, Titulaer, Maarten J., Verschuuren, Jan J., Gozzard, Paul, Lang, Bethan, Irani, Sarosh R., Sabater, Lidia, Graus, Francesc, Murray, Andrea, Chapman, Caroline J., and Neurology

Subjects
Adult, Male, SOX2, Sry-like high-mobility group box protein 2, Lung Neoplasms, Adolescent, SCLC, small-cell lung cancer, Paraneoplastic Syndromes, Lambert-Eaton myasthenic syndrome, SOX2, Sensitivity and Specificity, Article, Antibodies, Small-cell lung cancer, Young Adult, SDG 3 - Good Health and Well-being, ELISA, enzyme linked immunosorbent assay, Biomarkers, Tumor, Humans, Paraneoplastic, neoplasms, NMDA-R, N-methyl-d-aspartate receptor, Aged, Autoantibodies, Aged, 80 and over, LEMS, Lambert-Eaton myasthenic syndrome, PCD, paraneoplastic cerebellar degeneration, VGCC, voltage-gated calcium channel, SOXB1 Transcription Factors, LE, limbic encephalitis, Middle Aged, Small Cell Lung Carcinoma, humanities, respiratory tract diseases, OD, optical density, SSN, subacute sensory neuronopathy, Female, OMS, opsoclonus-myoclonus syndrome
Abstract

Antibodies to SOXB1 proteins in patients with paraneoplastic disorders are associated with small-cell lung cancer (SCLC), particularly in Lambert-Eaton myasthenic syndrome (LEMS). We aimed to establish if SOX2 antibodies could be used to identify SCLC and other tumours found in a range of paraneoplastic disorders and controls. SOX2 antibodies were detectable in 61% of patients with LEMS-SCLC, and in other paraneoplastic disorders, such as opsoclonus-myoclonus and paraneoplastic cerebellar degeneration, only when there was an underlying SCLC. SOX2 antibodies are specific (>90%) markers for SCLC, but are rarely found in patients with other tumours, whether neurological symptoms are present or not., Highlights • SOX2 antibodies only seen in small-cell lung cancer (SCLC), not other tumours • 61% of patients with Lambert-Eaton syndrome and SCLC have SOX2 antibodies. • Patients with paraneoplastic disorders only have SOX2 antibodies if SCLC is present.

Published
2019

36. Incidence and phenotypes of childhood-onset genetic epilepsies: a prospective population-based national cohort

Author

Symonds, Joseph D, primary, Zuberi, Sameer M, additional, Stewart, Kirsty, additional, McLellan, Ailsa, additional, O‘Regan, Mary, additional, MacLeod, Stewart, additional, Jollands, Alice, additional, Joss, Shelagh, additional, Kirkpatrick, Martin, additional, Brunklaus, Andreas, additional, Pilz, Daniela T, additional, Shetty, Jay, additional, Dorris, Liam, additional, Abu-Arafeh, Ishaq, additional, Andrew, Jamie, additional, Brink, Philip, additional, Callaghan, Mary, additional, Cruden, Jamie, additional, Diver, Louise A, additional, Findlay, Christine, additional, Gardiner, Sarah, additional, Grattan, Rosemary, additional, Lang, Bethan, additional, MacDonnell, Jane, additional, McKnight, Jean, additional, Morrison, Calum A, additional, Nairn, Lesley, additional, Slean, Meghan M, additional, Stephen, Elma, additional, Webb, Alan, additional, Vincent, Angela, additional, and Wilson, Margaret, additional

Published
2019
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39. Correction: Myasthenia gravis seronegative for acetylcholine receptor antibodies in South Korea: Autoantibody profiles and clinical features

Author

Park, Kee Hong, primary, Waters, Patrick, additional, Woodhall, Mark, additional, Lang, Bethan, additional, Smith, Thomas, additional, Sung, Jung-Joon, additional, Kim, Kwang-Kuk, additional, Lim, Young-Min, additional, Kim, Jee-Eun, additional, Kim, Byung-Jo, additional, Park, Jin-Sung, additional, Lim, Jeong-Geon, additional, Kim, Dae-Seong, additional, Kwon, Ohyun, additional, Sohn, Eun Hee, additional, Bae, Jong Seok, additional, Yoon, Byung-Nam, additional, Kim, Nam-Hee, additional, Ahn, Suk-Won, additional, Oh, Jeeyoung, additional, Park, Hyung Jun, additional, Shin, Kyong Jin, additional, and Hong, Yoon-Ho, additional

Published
2018
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40. Autoantibodies to neuronal surface antigens in thyroid antibody-positive and -negative limbic encephalitis

Author

Tuzun, Erdem, Erdag, Ece, Durmus, Hacer, Brenner, Tanja, Turkoglu, Recai, Kurtuncu, Murat, Lang, Bethan, Akman-Demir, Gulsen, Eraksoy, Mefkure, and Vincent, Angela

Subjects
Care and treatment, Physiological aspects, Genetic aspects, Research, Risk factors, Health aspects, Autoantibodies -- Health aspects, Potassium channels -- Physiological aspects -- Genetic aspects -- Research, Encephalitis -- Diagnosis -- Risk factors -- Genetic aspects -- Care and treatment -- Research
Published
2011

41. Lambert-Eaton syndrome IgG inhibits transmitter release via P/Q Ca2+ channels

Author

Spillane, Jennifer, Ermolyuk, Yaroslav, Cano-Jaimez, Marife, Lang, Bethan, Vincent, Angela, Volynski, Kirill E., and Kullmann, Dimitri M.

Subjects
Adult, Male, Mice, Knockout, Neurons, Action Potentials, Middle Aged, Calcium Channel Blockers, Synaptic Transmission, Article, Exocytosis, Lambert-Eaton Myasthenic Syndrome, Mice, Calcium Channels, N-Type, Immunoglobulin G, Animals, Humans, Female, Calcium Channels, Aged, Autoantibodies, Fluorescent Dyes
Abstract

Objective: To determine whether immunoglobulin G (IgG) from patients with Lambert-Eaton myasthenic syndrome (LEMS) decreases action potential–evoked synaptic vesicle exocytosis, and whether the effect is mediated by P/Q-type voltage-gated calcium channels (VGCCs). Methods: IgG was obtained from 4 patients with LEMS (3 males, 1 female), including 2 patients with lung malignancy. Antibodies against P/Q-type VGCCs were detected in all 4 patients, and against N-type VGCCs in 2. We incubated neuronal cultures with LEMS IgG and determined the size of the total recycling pool of synaptic vesicles and the rate of action potential–evoked exocytosis using fluorescence imaging of the amphiphilic dye SynaptoRed C1. Pooled IgG from healthy volunteers was used as a control. We repeated the experiments on synapses lacking P/Q-type calcium channels from a Cacna1a knockout mouse to determine whether these channels account for the pathogenic effect of LEMS IgG. Results: LEMS IgG had no effect on the total recycling pool size but significantly reduced the rate of action potential–evoked synaptic exocytosis in wild-type neurons when compared with neurons treated with control IgG. In contrast, LEMS IgG had no effect on the rate of synaptic vesicle exocytosis in neurons lacking P/Q-type channels. Conclusions: These data provide direct evidence that LEMS IgG inhibits neurotransmitter release by acting on P/Q-type VGCCs.

Published
2016
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42. Neuronal antibodies in pediatric epilepsy:Clinical features and long-term outcomes of a historical cohort not treated with immunotherapy

Author

Wright, Sukhvir, Geerts, Ada T., Jol-Van Der Zijde, Cornelia Maria, Jacobson, Leslie, Lang, Bethan, Waters, Patrick, Van Tol, Maarten J.D., Stroink, Hans, Neuteboom, Rinze F., Brouwer, Oebele F., and Vincent, Angela

Abstract

Objective In autoimmune encephalitis the etiologic role of neuronal cell-surface antibodies is clear; patients diagnosed and treated early have better outcomes. Neuronal antibodies have also been described in patients with pediatric epilepsy without encephalitis. The aim was to assess whether antibody presence had any effect on long-term outcomes in these patients. Methods Patients (n = 178) were recruited between 1988 and 1992 as part of the prospective Dutch Study of Epilepsy in Childhood; none received immunotherapy. Healthy age-matched bone-marrow donors served as controls (n = 112). All sera were tested for serum N-methyl-d-aspartate receptor (NMDAR), alpha amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, leucine rich glioma inactivated 1, contactin associated protein like 2 (CASPR2), contactin-2, glutamic acid decarboxylase, and voltage gated potassium channel (VGKC)-complex antibodies by standard techniques. No cerebrospinal fluid (CSF) samples were available. Results were correlated with clinical data collected over 15 years. Results Seventeen patients (9.5%) were positive for VGKC complex (n = 3), NMDAR (n = 7), CASPR2 (n = 4), and contactin-2 (n = 3), compared to three (3/112; 2.6%) healthy controls (VGKC complex [n = 1], NMDAR [n = 2]; p = 0.03; Fisher's exact test). Titers were relatively low (≤1:100 for cell-surface antibodies), but 8 (47%) of the 17 positive samples bound to the surface of live hippocampal neurons consistent with a potential pathogenic antibody. Preexisting cognitive impairment was more frequent in antibody-positive patients (9/17 vs. 33/161; p = 0.01). Fourteen antibody-positive patients were treated with standard antiepileptic drugs (AEDs); three (17%) became intractable but this was not different from the 16 (10%) of 161 antibody-negative patients. In 96 patients with available follow-up samples at 6 and/or 12 months, 6 of 7 positive antibodies had disappeared and, conversely, antibodies had appeared for the first time in a further 7 patients. Significance Neuronal antibodies were found at low levels in 9.5% of patients with new-onset pediatric epilepsy but did not necessarily persist over time, and the development of antibodies de novo in later samples suggests they could be due to a secondary response to neuronal damage or inflammation. Moreover, as the response to standard AEDs and the long-term outcome did not differ from those of antibody-negative pediatric patients, these findings suggest that routine neuronal antibody testing is unlikely to be helpful in pediatric epilepsy. However, the higher incidence of preexisting cognitive problems in the antibody-positive group, the CASPR2 and contactin-2 antibodies in 7 of 17 patients, and the binding of 8 of 17 of serum samples to live hippocampal neurons suggest that neuronal antibodies, even if secondary, could contribute to the comorbidities of pediatric epilepsy.

Published
2016

43. Myasthenia gravis seronegative for acetylcholine receptor antibodies in South Korea: Autoantibody profiles and clinical features

Author

Park, Kee Hong, primary, Waters, Patrick, additional, Woodhall, Mark, additional, Lang, Bethan, additional, Smith, Thomas, additional, Sung, Jung-Joon, additional, Kim, Kwang-Kuk, additional, Lim, Young-Min, additional, Kim, Jee-Eun, additional, Kim, Byung-Jo, additional, Park, Jin-Sung, additional, Lim, Jeong-Geon, additional, Kim, Dae-Seong, additional, Kwon, Ohyun, additional, Sohn, Eun Hee, additional, Bae, Jong Seok, additional, Yoon, Byung-Nam, additional, Kim, Nam-Hee, additional, Ahn, Suk-Won, additional, Oh, Jeeyoung, additional, Park, Hyung Jun, additional, Shin, Kyong Jin, additional, and Hong, Yoon-Ho, additional

Published
2018
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49. MOG cell-based assay detects non-MS patients with inflammatory neurologic disease

Author

Waters, Patrick, primary, Woodhall, Mark, additional, O'Connor, Kevin C., additional, Reindl, Markus, additional, Lang, Bethan, additional, Sato, Douglas K., additional, Juryńczyk, Maciej, additional, Tackley, George, additional, Rocha, Joao, additional, Takahashi, Toshiyuki, additional, Misu, Tatsuro, additional, Nakashima, Ichiro, additional, Palace, Jacqueline, additional, Fujihara, Kazuo, additional, Leite, M. Isabel, additional, and Vincent, Angela, additional

Published
2015
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